Bovine spongiform encephalopathy (BSE) is a transmissible, neurodegenerative and fatal disease of the family of transmissible spongiform encephalopathies (TSEs) which affects the brains of cattle. Various hypotheses have been advanced to explain the emergence of the pathogenic agent, such as its spontaneous presence in cattle whose carcasses were subsequently introduced into the food chain or its entry into this chain via the intermediary of sheep carcasses affected by a similar disease, scrapie.

Diagnosed for the first time in the United Kingdom in 1986, the "mad cow disease" took on epidemic proportions and finally posed a genuine public health problem following the discovery of a possible link between BSE and its human variant, Creutzfeldt-Jakob Disease, first diagnosed in 1996. By 1 December 2003 a total of 151 confirmed or suspected cases were registered in the European Union, mainly among young people. Most of the cases occurred in the United Kingdom (143), some in France (6), Ireland (1) and Italy (1). Without doubt this disease has also severely undermined consumer confidence and revealed the limits of the Community legislative framework.

Since 1997 the European Commission has thus committed itself to completely overhauling food safety legislation. In particular, the Directorate-General "Health and Consumer Protection" has been responsible since September 1997 for measures designed to protect public health and to guarantee food safety.

The current context

Since the early 1990s Community policy in the field of veterinary monitoring has become increasingly vigilant following the appearance in the United Kingdom of the first cases of BSE. The European Commission has adopted a large number of emergency measures to prevent the spread of this disease in the other Member States. It has prohibited any exportation from the United Kingdom of cattle and beef products and required the systematic slaughtering of herds in which a case of BSE has been diagnosed. Since January 2001 the ban on using "animal meal" in animal feeding has been in force throughout the territory of the European Union.

Currently, despite the detection of new cases of disease as a result of systematic checks, the BSE crisis is clearly on the wane. The number of BSE cases detected (more than 182 000 cases since 1986 in the United Kingdom and more than 3 800 in other countries, including 13 Member States) is steadily decreasing, in particular thanks to the improvement of the situation in the United Kingdom. In some Member States this number has nevertheless increased because of the systematisation of detection tests since July 2001. Arguably, the discovery of a number of cases in countries which practise active monitoring is more reassuring than the absence of cases notified in countries in which monitoring is unsatisfactory. Besides, in October 2002 France became the last Member State to lift the ban on British beef, whose exportation has again been permitted since August 1999 in the framework of the DBES (Date Based Exportation System) established at the time.

In the framework of Community law, all the emergency measures adopted are now being replaced by basic legislation which reinforces the rules governing the prevention, control and eradication of all transmissible spongiform encephalopathies (TSEs). This is the role of Regulation (EC) No 999/2001 which, by defining clear and uniform rules governing the entire food chain, should enable professionals and consumers to look to the future in a more serene manner. It goes without saying that in the light of enlargement and the adoption of the Community acquis by the future Member States, this new Regulation will play a decisive role in the field of animal health.

Adopted in May 2001, Regulation (EC) No 999/2001 [Official Journal L 147 of 31.05.2001] of the European Parliament and of the Council now constitutes the fundamental legal basis for all legislative instruments relating to BSE. In compliance with the general principles of food safety it consolidates previously scattered legislation of an overly sectorial nature. The Regulation is based on the latest scientific opinions and the recommendations of competent international organisations (World Health Organisation, Food and Agricultural Organization of the United Nations, International Office for Epizootic Diseases). Due to the magnitude of the BSE crisis, the Regulation is based on Title 152 ("Public Health") of the Treaty establishing the European Community. It lays down measures governing the prevention, control and eradication of transmissible spongiform encephalopathies (TSEs) in bovine, ovine and caprine animals.

Notably the Regulation lays down:

the procedure, criteria and categories making it possible to define the BSE status of the Member States and third countries;

the nature and treatment of specified risk material (SRM);

the active monitoring system:

prohibitions concerning animal feeding.

Classification of Member States as regards BSE

At the Commission's initiative, the Scientific Steering Committee (SSC) of the European Union has prepared a "geographic risk assessment" of the BSE situation in the EU Member States and in third countries. The method used to carry out this assessment, which has lasted more than two years, is based on the dossiers submitted by the countries concerned in response to a Commission Recommendation of 1998. This recommendation defines the information needed to carry out such an assessment. It notably concerns imports of live bovines and meat and bone meal from the United Kingdom and other BSE affected countries, the feeding of mammalian meat and bone meal to ruminants, animal by-product rendering practices, the use of specified risk material (SRM) and the use of meat and bonemeal to feed ruminant animals.

The SSC has defined four categories on the basis of the geographic risk assessment :

Level I: countries in which BSE is highly unlikely;

Level II: countries in which BSE is unlikely but not excluded;

Level III: countries in which BSE is likely but not confirmed, or confirmed but at a low level;

Level IV: countries in which BSE has been confirmed at a large scale.

The SSC has evaluated or is still evaluating the status of third countries in respect of the geographical risk of BSE as though they were EU Member States. This geographical risk of BSE is the only determining factor which can specify the level of protection required. Up to now, the existence of BSE has been judged highly unlikely in the 17 following third countries: Argentina, Australia, Botswana, Brazil, Chile, Costa Rica, Iceland, Namibia, Nicaragua, the French overseas territory New Caledonia, New Zealand, Panama, Paraguay, Salvador, Singapore, Swaziland, Uruguay, Vanuatu. Hence these countries have been exempted from restrictions. Currently, no EU Member States benefit from a similar status. Besides, the WHO rules are respected in the sense that the Union notifies its BSE measures to third countries.

Regulation (EC) No 999/2001, which entered into force on 1 July 2001, provides for the classification of the countries in five BSE risk categories in line with the code imposed by the OIE. Currently the Commission is preparing this classification, which involves two stages: an initial risk assessment taking into account specific predefined factors and a second assessment on the basis of additional criteria. Pending the determination of the BSE status, transitional measures apply. They are based on the classical geographical BSE risk assessment as undertaken by the Scientific Steering Committee.

Specified risk material (SRM)

Scientists very soon realised that certain tissues are very likely to carry the infectious agent and transmit BSE. By systematically eliminating them from the human and animal food chain, it seems that it is possible to reduce the risk of transmission appreciably.

As from April 1996, a group of experts set up by the World Health Organisation (WHO) recommended that all animals, as well as products derived from them, which have shown signs of transmissible spongiform encephalopathy be excluded from the human and animal food chain. In the same year the Scientific Veterinary Committee (SVC) also recommended that, pursuant to the precautionary principle, these tissues or "specified risk material" (SRM) be systematically withdrawn and destroyed in order to eliminate any risk of recycling TSE agents.

SRM is currently the subject of transitional measures. These are applicable pending the adoption of a Community decision concerning the BSE classification of the country under consideration. Besides establishing procedures for eliminating specified risk material, Regulation (EC) No 999/2001 also defines their specific nature:

the skull excluding the mandible but including the brain and eyes, the vertebral column excluding the vertebrae of the tail, the transverse processes of the lumbar and thoracic vertebrae and the wings of the sacrum, including dorsal root ganglia, and spinal cord of bovine animals aged over 12 months, and also the tonsils, the intestines from the duodenum to the rectum and the mesentery of bovine animals of all ages;

the skull including the brain and eyes, the tonsils, the spinal chord of ovine and caprine animals aged over 12 months or which have a permanent incisor erupted through the gum, and the spleen and ileum of ovine and caprine animals of all ages.

Besides the SRM referred to above, the following tissues from the United Kingdom and Portugal (with the exception of the Autonomous Region of the Azores) must be designated as such: the entire head, excluding the tongue, but including the brain, eyes and trigeminal ganglia; the thymus, spleen and spinal cord of bovine animals aged over six months.

A derogation is possible to allow the use of the vertebral column and dorsal root ganglia from bovine animals born, raised and slaughtered in Member States where a scientific assessment suggests that BSE is highly unlikely. The same applies to bovine animals from Member States which have declared cases of BSE born after the effective ban on the feeding of mammalian protein to ruminants. To benefit from this derogation, the United Kingdom, Portugal and Sweden must produce scientific evidence. The derogation is not applicable to bovine animals aged over 30 months from the United Kingdom or Portugal (with the exception of the Azores).

The bones of bovine, ovine and caprine animals must not be used for the production of mechanically recovered meat. Meat from the head and the vertebral column of bovine animals aged over 12 months and the tongue of bovine animals of all ages must be collected using specific methods.

Specified risk materials are removed in slaughterhouses, cutting establishments in the case of the vertebral column of bovine animals and, where appropriate, the approved establishments referred to in Regulation (EC) No 1774/2002 concerning animal by-products not intended for human consumption. On removal, all SRM and by-products thereof must be stained with a dye or marker. They must be used and destroyed in accordance with Regulation (EC) No 1774/2002. Member States must carry out frequent official checks to ensure that these provisions are being applied and that measures are adopted to avoid contamination all along the production line.

Active monitoring system

The BSE incubation period is approximately four to five years. During this period, bovine animals exposed to the causative agent do not present any symptoms. In order to monitor the trends of the epidemic, an active monitoring system is thus necessary. Hence the commission must approve and finance national monitoring programmes for TSEs and scrapie together with specific measures (purchase of screening kits, slaughtering, genotyping) in the Member States as well as Cyprus, Estonia, Malta and Slovenia.

Each Member State must carry out an annual programme for monitoring BSE and scrapie. Annex III sets out the monitoring system to be applied for these two diseases. Since January 2001, besides the compulsory controls of all the animals displaying signs compatible with a TSE, the rapid post mortem screening must be practised on:

all bovine animals aged over 24 months intended for human consumption and subjected to emergency slaughtering;

all bovine animals aged over 30 months, slaughtered in normal conditions for human consumption or as part of a BSE eradication campaign;Sweden may derogate from this rule by performing the tests only on a random sample of animals;

all bovine animals aged over 24 months not intended for human consumption, found dead, or slaughtered (but not in connection with an epidemic such as foot-and-mouth disease);Member States may derogate from these provisions in remote areas where the animal population density is low and there are no arrangements for the collection of dead animals. They must inform the Commission accordingly. The derogation may not be applied to more than 10 % of the Member State's bovine animal population;

all bovine animals which are emergency slaughtered or declared sick following ante mortem screening;

all bovine animals aged over 42 months, born after 1 August 1996;

a random sample comprising at least 10 000 bovine animals per year;

all ovine and caprine animals not intended for human consumption aged over 18 months or with more than two permanent incisors erupted through the gum, which have been found dead or have been slaughtered (though not as part of an eradication campaign);Member States must carry out screening tests based on a representative sample, giving priority to animals found dead on the farm;

since 1 October 2003, animals aged over 12 months with a permanent incisor erupted through the gum, on the basis of a representative sample;

all other animals;Member States may carry out monitoring programmes in respect of animals used for dairy production, animals derived from herds infected with TSE or derived from TSE infected females, animals which have consumed potentially contaminated feed.

The Member States must submit to the Commission an annual report notably containing information on the number of suspected cases by animal species which have been subjected to movement restrictions.

A total of over 8.5 million bovine animals were tested in 2001 and over 10 million in 2002. Currently the Commission is co-financing the test programme in the amount of 10.5 euros per test. Further information on the number of tests carried out and the number of detected cases can be found at the European Commission's "Food Safety" site.

Prohibitions concerning animal feeding

Very soon after the emergence of BSE, British scientists suspected that the consumption by bovine animals of meat and bone meal was responsible for the spread of the epizootic. Since July 1998, the United Kingdom has banned the use of mammalian proteins in the feeding of ruminant animals. This prohibition entered into force in the European Union in June 1994, the date of adoption of the first Community decision in this connection [Decision 94/381/EC, repealed by Regulation (EC) No 1326/2001].

Regulation (EC) No 999/2001 lays down measures concerning animal feeding and upholds the ban on the use of animal proteins and feedingstuffs containing such proteins in the feeding of ruminants. Livestock, with the exception of fur-producing carnivores, must not be fed with processed animal protein, gelatine of ruminant origin, blood products, hydrolysed protein, dicalcium phosphate and tricalcium phosphate of animal origin.

These prohibitions do not apply to the use of the following feedingstuffs and proteins which, where appropriate, have been processed in accordance with the provisions of Regulation (EC) No 1774/2002 on animal by-products not intended for human consumption:

in the feeding of non-ruminants: fishmeal, hydrolised proteins derived from non-ruminants and ruminant hides and skins, dicalcium phosphate and tricalcium phosphate;

in the feeding of ruminants: milk, milk-based products and colostrum, eggs and egg products, gelatine derived from non-ruminants;

in the feeding of fish: blood products and blood meal derived from non-ruminants.

Since 1 November 2003, Member States have been required to provide the other Member States and the Commission with an up-to-date list of slaughterhouses approved by the European Union which do not slaughter ruminants. They must also forward a list of establishments authorised to produce the feedingstuffs and proteins referred to above.

Exports to third countries of processed animal proteins from ruminants and of products containing such proteins must comply with the legislation in force in the Community territory. The third country of destination must provide a prior undertaking in writing to use and/or export products taking account of the intended final use. The Member State authorising the export must inform the other Member States and the Commission.

The legislation on animal waste enhances the effectiveness of the ban on "animal meal". In this connection, Regulation (EC) No 1774/2002 [Official Journal L 273 of 10.10.2002] on animal by-products not intended for human consumption entered into force in May 2003. It lays down strict control measures concerning the collection, transport, storage, processing and use or disposal of 16 million tons of material unsuitable for consumption produced each year. It also prohibits intra-species recycling in order to exclude cannibalism.

Research and development on BSE

The European Commission has been financing research on TSEs since 1990. This research activity was rapidly expanded following the announcement by the UK Government in March 1996 that 10 cases of new-variant Creutzfeldt-Jakob Disease might be linked to exposure to BSE.

In April 1996, the Commission invited Professor Charles Weissmann to chair the group responsible for drawing up an inventory of existing knowledge in this area and to suggest priorities for research. Published in October of the same year, Professor Weissmann's report was the basis for a communication from the Commission to the Council [COM(96)582 final] proposing a TSE plan of action. This plan of action also took into account the recommendations of the Multidisciplinary Scientific Committee and ongoing research at national and Community level. It included two levels:

the coordination of activities between the Member States, with a view to harmonising data collection and diagnostic criteria;

a specific call for proposals intended to stimulate research at Community level.

The first of the three calls for proposals in the framework of this initiative was launched in December 1996, the last in March 1998. A total of 54 projects have been approved and have received EUR 50.7 million altogether in the framework of the BIOMED, BIOTECH and FAIR programmes. Hence the plan of action made it possible to rally experts in numerous scientific disciplines and from more than 120 laboratories in all the Member States and associated countries.

In November 2000, the Research Council requested the Commission to establish a TSE Expert Group with a mandate to examine the state of TSE research, encourage the exchange of scientific information between research teams, identify ongoing research activities which need to be strengthened, as well as new research activities which need to be launched. The Group is composed of representatives nominated from the Member States, Associated Countries, some members of the TSE/BSE ad hoc group of the Scientific Steering Committee and some co-ordinators of EU research projects. In April 2002, with an eye to enlargement, it was expanded to include members from Central and Eastern European States.

The plan of action [COM(96) 582 final] notably included the objective to expand and regularly update the inventory of TSE research activities in Member States. The Commission has published a specific communication relating to this [COM(2001) 323 final].

In April 2001 an analysis of the most recent version of the Weissmann report resulted in the launch of a specific call for TSE proposals, intended to fill the gaps in the European TSE research effort. A total of 15 new TSE projects were commissioned from this call with support totalling EUR 21 million. All in all, the fifth framework programme for research and development (1998-2002) will have supported 26 projects to a tune of almost 30 million euros: 11 research projects financed following general calls for proposals and 15 on the basis of the specific call for proposals of 2001.

The sixth framework programme for research and development (2002-2006) includes priorities for actions concerning "Food quality and risks to health" and has a budget of 685 million euros allocated to this. The transversal objective is to establish the scientific and technological bases needed to produce and distribute safer, healthier and varied food. It is this strand which will participate in financing research into TSEs.

International dimension and enlargement

In the accession negotiations all candidate countries have committed themselves to fully comply with the Community rules on combating BSE. They have made substantial progress: all of them are already removing the specified risk materials from the food chain and majority of them are testing all healthy cattle aged over 30 months. In 2003, 40 % of the veterinary inspections of the Food and Veterinary Office (FVO) will take place in future Member States with a view to verifying the measures taken with regard to food safety, including those designed to combat BSE. In addition, the Commission cofinances screening kits and provides its technical assistance in the framework of the Phare programme.

At international level the European Union has developed close cooperation with the following international organisations: the World Health Organisation (WHO), the Food and Agricultural Organisation of the United Nations (FAO) and the International Office on Epizootic Diseases (OIE).

Since 1991 the WHO has organised 11 scientific consultations on issues concerning human and animal TSEs. The group of independent specialists set up by the WHO continues to update knowledge on the basis of new scientific information. A neutral forum has been set up to examine, evaluate and discuss scientific questions concerning TSEs. The WHO encourages research in this field by publishing a list of 11 priority areas, notably on early diagnosis and epidemiology. Finally, it promotes systematic monitoring of the Creutzfeldt-Jakob Disease and its variants in order to get a clearer picture of its geographical breakdown throughout the world. Consult the BSE section at the WHO site.

A BSE consultative committee was set up at the FAO in 1990. In cooperation with the WHO and the OIE it regularly convenes scientific experts who review research developments in this area.

Based in Paris, the International Office on Epizootic Diseases (OIE) has been the international organisation on animal health since 1924. It aims to ensure transparency as regards the animal diseases situation throughout the world, notably BSE. It collects, analyses and disseminates scientific veterinary information and provides its international expertise with a view to combating animal diseases. It guarantees the food safety of world trade by drafting health rules on the international trade of animals and their products.

See also

Further information

- European Commission: Food Safety site of the Directorate-General "Consumer Protection";

- Site of the Council of the European Union ("Employment, Social Policy, Health and Consumers", "Agriculture and Fisheries");